Connective Tissue Disease
Table 1: Assessment Methods for Diagnosis and Follow-up of Organ Involvement in Systemic Sclerosis Organ Involvement
Diagnosis 6 months Skin Renal function assessment Gastrointestinal MRSS score
Creatine clearance Urinary protein excretion Echo doppler ultrasound Oesophageal manometry Barium study
Heart
Glucose hydrogen breath test 24-hour pH monitoring Standard ECG
24-hour Holter ECG Echocardiography
Pulmonary ILD
PAH
Vascular Micro Macro
Pulmonary function test, HRCT
Echocardiography, pulmonary function test,
Six-minute walking test Nailfold capillaroscopy
Angio MRI Arterial echo Doppler, IMT thickness
BAL = bronchoalveolar lavage; ECG = electrocardiogram; GFR = glomerular filtration rate; HRCT = high-resolution computed tomography; ILD = interstitial lung disease; IMT = intima-media thickness; MRI = magnetic resonance imaging; MRSS = modified Rodnan skin thickness score; PAH = pulmonary arterial hypertension; SPECT = single photon emission computed tomography.
is altered in patients with myocardial structural impairment, even if asymptomatic; proBNP levels might be measured at least once a year to monitor any possible heart modification.
Pulmonary Involvement
There are two principal manifestations of lung involvement in SSc: interstitial lung disease (ILD) and pulmonary vascular disease with pulmonary arterial hypertension (PAH). Differentiation between ILD, PAH and other causes of dyspnoea in patients with SSc can be clinically difficult. Respiratory symptoms can be non-specific, and early stage ILD can develop undetected.
Pulmonary function tests play a major role in the investigation of dyspnoea and the evaluation of pulmonary complications. Impaired diffusion capacity of the lung for carbon monoxide (DLCO) is an early marker of both ILD and PAH, and correlates with the severity of underlying disease in both instances.32,33
three years is closely associated with increased mortality.34
A decrease in DLCO over Lung
involvement is defined as normal when forced vital capacity (FVC) and DLCO are over 80% of the predicted values adjusted for age, sex and height. It has been proposed that disease severity in patients with SSc be defined as mild, moderate or severe when these measurements are 70–79, 50–69 and under 50% of the predicted values, respectively.35 test is mandatory.
The value of bronchoalveolar lavage (BAL) in the definition of alveolitis in SSc-related ILD has been investigated by Goh et al.38
who identified
an association between neutrophilia on BAL and early mortality. However, no associations were observed between neutrophilia, eosinophilia or lymphocytosis on BAL and the rapidity of functional deterioration or progression-free survival. BAL is not recommended routinely but may be useful in selected cases, especially for differential diagnosis with other diseases or infections.
For SSc lung assessment, induced sputum has been proposed as an alternative method.39
It may allow a non-invasive assessment of cell
composition in airway fluid and may contribute to the better understanding of upper/middle airway inflammation, but future studies are needed to verify whether this method can replace invasive procedures for detecting and monitoring lung inflammation in SSc.
A biannual assessment of a pulmonary function
The introduction of high-resolution CT (HRCT) has significantly increased the sensitivity of X-ray diagnosis, particularly in terms of the early detection of ground glass, and HRCT is now the gold standard to assess ILD. On HRCT, the extension of ILD correlates with a worse prognosis in terms of increased mortality and more rapid deterioration of lung function tests. An extension of ILD to more than 20% of lung volume (extensive disease) is correlated with poorer prognosis, while patients with <20% of lung involvement (mild
40
Recently, lung ultrasound has identified comets, echographic signs of ILD that are more frequent in the diffuse than the limited form of SSc and correlate with HRCT-derived assessment of lung fibrosis.40
Lung
comets may represent a simple, radiation-free hallmark of pulmonary fibrosis of potential diagnostic and prognostic value but the technique awaits confirmation and standardisation.
For detection of PAH, echocardiography is recommended as a biannual screening tool and is mandatory in all SSc patients, even in the absence of any symptoms.41
In the case of detection of abnormal
values, right heart catheterisation is required to confirm the diagnosis of PAH.42
In addition, the combination of elevated echocardiographic EUROPEAN MUSCULOSKELETAL REVIEW
HRCT has to be performed annually to detect variations of ILD extension, even if this choice may be criticised for the high X-ray exposure of the patients.37
disease) have no increase in mortality compared with SSc patients without ILD.36
Follow-up 12 months Occasionally Ultrasound, durometer Isotope GFR, renal biopsy
Upper endoscopy, endoanal MRI, Video capsule endoscopy Anorectal manometry
MRI, heart rate variability QT dispersion,
Invasion electrophysiological studies Myocardial biopsy, SPECT Coronary angiography
BAL, induced sputum, lung comets Cardiac catheterisation
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